1-48-08 Recurrent facial palsy in HNPP

S64

Peripheral

Nerve Disease other than Immunological

variability of repeat measures and correlation with sural nerve conduction parameters. Research Design and Methods: 152 patients with diabetic peripheral neuropathy, received electodiagnostic evaluation and quantitative VPT testing with the Vibratron II and the Horwell Neurothesiometer. Forty-two patients returned for repeat nerve conduction studies and VPT testing with both types of equipment on 3 separate occasions. Reeulb: The variability of repeat testing for the vibratron was 33% and for the neurothesiometer was 7%. This variability compares to that of sural nerve conduction velocity of 2% and sural nerve amplitude of 8% in this series of patients. VPT from the vibratron correlated with the sural nerve amplitude with R2 0.072 and 0.137. VPTfrom the neurothesiometer gave R2 values of 0.272 and 0.268. Correlation of VPT with sural netve conduction velocity for the vibratron gave R2 of 0.120 and 0.161. For the neurothesiometer, the values were 0.307 and 0.283. All correlations were highly significant. Conclusion: We conclude that VPT determined with the neurothesiometer is less variable than with the vibratron and more reflective of peripheral nerve function. Our results indicate that the neurothesiometer can be used reliably in clinical research trials.

l-48-04 u

Drug choice of oral medication syndrome

Ming-Hong Chang. Hospital-Kaohsiung,

Section of Neurolog)! Kaohsiung, Taiwan

Veterans

in carpal tunnel General

Purpose: Conservative treatment is variable for most patients with mild to moderate carpal tunnel syndrome (CTS). So far, there is no adequate study of the utility of the common oral medication such as non-steroid anti-inflammatory drug (NSAID), diuretic or oral steroid. Therefore, we want to evaluate the effectiveness of NSAID, diuretic and oral steroid in the treatment of CTS. Materials and Methods: Patients with clinical symptoms and signs of CTS, confirmed by standard electrc-diagnosis, comprise the patients group and then enter into this investigation. The baseline assessments include a standardized symptoms questionaire. The questionaire use a 10 point ordinal scale on which symptoms are rated from 0 (no symptoms) to 10 (severe) and patients rate pain, numbness, paresthesia, clumsiness and nocturnal awakening. The total score is termed global symptoms score. Following the baseline assessment, patients are randomized to 1) a P-week treatment period with prednisolone 20 mg daily followed by a P-week of 10 mg daily, 2) 4-week treatment with diuretic,. 3) 4-week treatment with NSAID-SR and 4) placebo. Follow up assessment on 2 and 4 weeks are identical. Then the change of global scores is analyzed to determine the statistical difference. Results and Conclusion: The baseline and the following 2 week and 4 week assessments in placebo, NSAID-SR, and diuretic groups do not show any significance in global score reduction. However, in the steroid group, the mean score is decreased from baseline 27.9 f 6.9 to 4-week 10 f 7.4 which is significant compared with other groups. Therefore, it is more appropriate for most patients with mild to moderate CTS to receive conservative treatment in which oral steroid is the only oral medication in alleviating symptoms of CTS.

1 l-48-05

1 Clinical, eiectrophysioiogicai, and genetic studies on hereditary neuropathy with liability to pressure palsies (HNPP)

Y.C. Chang, CC. Huang, SF. Sung, J.S. Peng, P.F. Chance, R.C. Chen, ST. Hsieh. Department of Neurology; National Taiwan lJnive&y Hospital, Taipei, Taiwan, Department of Neurofogyl Children’s Hospital of Philadelphia, PA, USA Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder with recent genetic characterization. We performed extensive studies on a large kindred of 3 generation in Taiwan, including neurologic, electrophysiological and genetic investigation. Fifteen of the members were available for evaluation. The proband suffered from repeated peroneal nerve palsies. Eleven of them (73%) were affected symptomatically or subclinically. Electrophysiological studies revealed a pattern of entrapment neuropathy involving multiple sites. The most frequent lesion was in the median nerve, the carpal tunnel syndrome. Three of the members (20%) examined had a generalized neuropathy. The genetic defect was further characterized by newly developed method to demonstrate a novel iunctional fragment of 7.8 kb in size, cbnsistent with a 1.5 Mb deletion on tie short arm if chromosome 17, 17~11.2-12 (Hum Mol Genet 1996; 5: 745). These findings suggest clinical suspicion and genetic investigation help in identifying the etiology-f peripheral neuropathy.

l-48-06 Young-chul Severance

Mediated

Clinical significance of residual latency and terminal latency index in diabetic patients Choi, Joon-shik Moon. Department of Neurolog) Yongdong Hospital, Yonsei University College of Medicine, Seoul, Korea

BackgroundlObjectlve: Although peripheral neuropathy in diabetic patients can be easily recognized when fully developed, it frequently present a difficult diagnostic challenge in its early stages. The electrophysiologic study, especially nerve conduction study (NCS), is one of the most objective and qualitative method for the evaluation of peripheral neuropathy. However, some patients have infrequently shown normal ranges of NCS even though they may have definite symptoms and signs of diabetic neuropathy. The authors electrophysiologically evaluated distal motor nerve dysfunction of upper extremities in diabetic patients. Design/Methods: The terminal latency (TL), the motor newe conduction velocity over wrist-elbow segment (NCV) and the residual latency (RL) on median and ulnar newes were measured in 20 diabetic patients with symptomatic peripheral polyneuropathy who were electrophysiologically normal, and the results were compared with those of 20 normal subjects. RL was calculated by dividing the distance between the cathode and active electrode by motor NCV and subtracting the quotient from the motor terminal latency. Results: The TL, NCV and RL in median motor nerves were 3.44 f 0.99 (msec), 56.51 f 5.23 (m/s), and 2.55 f 0.29 (msec) in diabetic patients, and 3.25 f 0.34, 59.13 f 6.50 and 2.33 f 0.33, respectively. The TL, NCV and RL in ulnar motor newes were 2.57 f 0.28 (msec), 66.64 f 5.62 (m/s) and 1.66 f 0.30 (msec) in diabetic patients, and 2.37 f 0.39, 56.16 f 6.65 and 1.44 f 0.36, respectively. There is no significant difference in latency and velocity of median and ulnar newe between diabetic patients and normal controls. The mean residual latency of diabetic patients is significantly prolonged compared to the normal control (p < 0.05). Conclusions: The RL may be more sensitive and useful in confining early or mild diabetic motor neuropathy and particularly attractive because it does not require an additional stimulation of the nerve. preventing the patients from the additional discomfort resulted from that procedure.

l-48-07

N-hexane neuropathy as an occupational disease A clinical and neurophysiologicai study of four cases

P.E.S. Easaw.

Neurology

Clinic, General

Hospital,

Penang, Malaysia

The objective of this paper is to present the occurrence of n-hexane neuropathy as an occupational disease in a cable factory, its clinical characteristics, neurophysiological findings and prognosis. Four employees of a wire and cable factory were referred to this clinic with a history of progressive numbness of the hands and feet of a few months duration. They all had constant exposure to n-hexane for about two years. The physical findings were those of a sensorimotor peripheral neuropathy. Nerve conduction studies confirmed a combined axonal and demyelinating sensorimotor polyneuropathy. Toxicological studies of urine and blood were negative for n-hexane. Nevertheless removal from exposure resulted in gradual but full recovery in all patients. N-hexane, which is used in industry as a solvent and cleansing agent is a known cause of peripheral neuropathy. Circumstantial evidence definitely indicates this as the cause here. Recognition of this and removal from exposure resulted in full clinical recovery. The factory concerned has since discontinued the use of this chemical, and there has been no recurrence of similar symptoms amongst its employees. Exposure control measures must be strictly ensured In all industies using n-hexane.

1l-48-08

] Recurrent

facial palsy in HNPP

T.E. Poloni, F. Simonetti, F. Imbesi, E. Alfonsi, I.M. Merlo, S. Botti I, F. Taroni ‘, M. Ceroni. Dept Neuroscience Foundation IRCCS C. Mondino, Pavia, ItaQ ’ Neurological institute C. Besta, Milano, ltaly Hereditary neuropathy with liability to pressure palsies (HNPP) is a frequent and underdiagnosed neuropathy due to a macrodeletion in the peripheral mielin protein gene-22 (PMP 22). Involvement of cranial newes in HNPP has been recently excluded even if previous descriptions reported facial and trigeminal involvement. Recurrent facial palsy has never been reported as an onset symptom. A 20-year-old man arrived to our attention with a previous history of idiopatic recurrent facial palsy. He was admitted to our Neurological Department because of a P-month history of left foot-drop. Previously he had expedenced a transient weakness of the extension of his right hand. EMG revealed a diffuse slowing of conduction velocities, especially across entrapment sites. Blink reflex latencies was prolonged bilaterally. The deletion of the PMP 22 gene (17~11, 2-12 segment) was found in our patient. His father had occasionally experienced only a transient weakness and numbness in the ulnar territory bilaterally. His 18-year old brother was asymptomatic. They both have the PMP 22 gene deletion and pathologic EMG findings, including alterations of the blink

Prevention of Neurological

reflex. The mother and the two sisters are not affected at clinical, genetic and EMG testing. We report a HNPP family in which the proband has had a recurrent facial palsy as first clinical manifestation. We want to underline the observation that cranial nerves, specially facial nerve, may be involved in HNPP.

Disorders

Major bleeding occurred in 13 patients (2%). Four major bleedings were intracerebral (two fatal), eight were gastrointestinal, and one was retinal. Fiftyeight patients (9%) dropped out, and 117 patients (17%) were withdrawn. Patients receiving adjusted-dose warfarin had the highest withdrawal ratio. The rate of primary and secondary events in the four treatment groups are currently analyzed. The results will be presented.

l-49-03

49

Prevention of Neurological Disorders

l-49-01

Cerebral function in workers previously exposed to metallic mercury at a chlorine alcali plant

K. Grunnet, I.P. Pedersen, J. Hansen, S. Mikkelsen. Clinic of Occupational Medicine, Hospifal of K6ge, DK-4600 KcTge, Denmark The aim was to investigate if a chronic effect on the central nervous system could be found in workers, who previously had been exposed to mercury vapours at a chlorine alcali plant. 67 workers were investigated. They had been employed l-30 years. The workforce was very stable and most of the heavier exposed persons had been supervised closely for mercury exposure over more than 10 years at air concentrations resulting in urine concentrations frequently exceeding 100 pg Hgil. A special exposure index was calculated for each worker. All workers were tested for present urine Hg-concentration and neurologitally computer tested for coordination ability, tremor in hand/fingers and balance. All filled in a questionnaire concerning employment, health data including CNS and PNS symptoms and life-style. The present mercury urine concentrations were all within the normal range, and there were no differences between subjects with low and high previous exposure with respect to questionnaire symptoms or performance in the computer tests. It is concluded that the biological limit value of 100 pg Hg/l urine seems to be sufficient to protect workers from chronic adverse effects from mercury vapour exposure.

Il-49

02

Fixed lowdose warfarin alone or combined with asdrin and aspirin alone versus adiusteddoss warfarin for stioke prevention in atria1 fibrillation. Second Copenhagen atrlal flbrlllation, aspirin and anticoagulation study (AFASAK 2)

A.L. Gullev, B.G. Koefoed, P. Petersen, T.S. Pedersen, J. Godtfredsen, E.D. Andersen. The AFASAKP Study Denmark

Background:

G. Boysen, Center, Copenhagen,

Non-valvular atrial fibrillation is associated with an overall risk of ischemic stroke of 4.5% per year. Adjusted-dose warfarin reduces the risk of stroke by 66% but is not considered ideal for elderly patients. Objective: To estimate the stroke preventive effect of 1) warfarin 1.25 mg daily, 2) warfarin 1.25 mg + aspirin 300 mg daily, 3) aspirin 300 mg daily, and 4) adjusted-dose warfarin (INR 2.0-3.0) in out-patients with chronic atrial fibrillation. Methods: AFASAKP was a randomized, one center trial. The primary event was a stroke (ischemic or haemormagic) or a systemic thromboembolic event. Transient ischemic attack, acute myocardial infarction and death not due to a primary endpoint were secondary events. Results: From May 1993 till October 1996, a total of 677 patients were included (median age of 74 (range 44-69)). A total of 46 patients (7%) had a primary event and 71 (11%) had a secondary event.

S65

Hereditary and genetic neurological opportunities for prevention

M. Ben Hamida, M. Zouari, N. Khouja, C. Ben Hamida, F. Hentati. INN /a Rabta, Tunis, Tunise

disorders A. Larnaout,

S. Belal,

Hereditary and genetic neurological disorders are heterogeneous. Prevention of these diseases is based on the progress and the power of current methods of linkage analysis positionnal cloning and identification of the gene product. Various techniques are used, depending on the pathophysiology and the gene functioning in each disease. The infantile and adult metabolic familial encephalopathies (aminoacidopathies storage diseases etc) muscular dystrophies (Duchenne-Becker, limb girdle musculair dystrophies), myotonic diseases, neuropathies (Charcot Marie Tooth diseases etc) and familial neurodegenerative diseases (Parkinson, Alzheimer, Friedreich, Huntington diseases, amyotrophic lateral sclerosis and motor neuron diseases, etc) will be reviewed. Study of mode of inheritance of the diseases, detection of heterozygotes, and detection of metabolic and enzymatic deficiency, are usefull for better treatment, and prevention. Ethical problems will be discussed.

1 49 04 L-2-J

Antioxidant

potential of green tea

P. Jovic, A. JoviEiC, B. Boskovic, T. Lepid. Central Clinical Laboratory, Neurology, Military Medical Academy Belgrade, Yugoslavia

Clinic of

Neutralizing free radical is an important step in the treatment of cerebral ischemia. The purpose of this work was to investigate antioxidant activity of China green tea, which has recently been used to the prevention of stroke. Extract from green tea was prepared by maceration using ethanol and water (7:3). Antioxidant activity of green tea vas determined spectrophotometrically by the inhibition of ABTS (2.2 - Azino - di - 3 - ethylbenzthiazoline sulphonate) oxidation, using kit from Randox, United Kingdom, and compared with antioxidant activity of ascorbic acid (10 mmol/l). The inhibition of ABTS oxidation with green tea extract was lOO%, as well as with ascorbic acid. It was concluded that green tea has a good antioxidant potential, speaking thus in favour of its rational use to the prevention in ischaemic brain damage, as shown by extensive, four-year study in Japan.

1 l-49-05

] Cross-cerebellum

E. Mendizabal.

School of Medicine,

atrophy and related disorders Buenos

Aires, Argentina

The anatomicopathological study of a case of cross-cerebellum atrophy (the first case submitted to a pathological study in our country) is shown. The clinical setting is not significant since hemiplegia is hidden by cerebellar symptoms. On the contrary, the anatomicopathological study is of great importance because it clarifies the complicated cerebral-cerebellar connections. We support the transneural or secondary theory, based on the fact that the origin of cerebellar lesions is in brain disorders that occurred inside the womb or in early childhood The atrophy of motor route has an effect on Varolius’ bridge cells and finally on Purkinje cells.